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Functional Studies on Lymphocytes from Two Siblings with Congenital Hypogammaglobulinaemia
Author(s) -
Tauris Palle,
Hansen Peter Wendelboe
Publication year - 1983
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1983.tb01455.x
Subject(s) - pokeweed mitogen , common variable immunodeficiency , antibody , immunology , b cell , t cell , thymidine , immune system , microbiology and biotechnology , dna synthesis , lymphocyte , biology , medicine , peripheral blood mononuclear cell , dna , in vitro , biochemistry , genetics
Two brothers with hypogammaglobulinaemia classified as common variable immunodeficiency (CVID) were investigated for distribution of peripheral blood lymphocyte (PBL) subpopulations, DNA synthesis and plaque‐forming cell (PFC) capability of pokeweed mitogen (PWM) activated autologous and allogenic cocultures. Both patients had a decreased absolute number of T cells and normal or elevated levels of surface immunoglobulin (SmIg) bearing cells. Isolated B cells cocultured with autologous or allogeneic 4000 r irradiated T cells responded subnormally to PWM monitored by the 3 H‐thymidine incorporation in microcultures whereas B cells cocultured with allogeneic untreated normal T cells proliferated normally. PBL from parallel macrocultures of unfractionated or T/B separated patients' cells were not able to produce plaques using a reversed haemolytic protein A assay. Addition of glucocorticoid to unfractionated PBL did not reverse the unresponsiveness. In allogeneic co‐cultures patients' untreated or 2000 r irradiated T cells induced a normal PFC response. Normal untreated T cells induced a reduced number of IgM‐ and IgG‐PFC from patients' B cells but this response was almost eliminated using irradiated normal T cells. These results demonstrate a primary B cell defect in the patients and indicate an impaired cooperation between patients' B and T cells. Activation of patients' B cells to Ig secretion requires the presence of proliferating T cells.